[Practical problems by implementation of vaccination recommendations]. / Praktische Probleme bei der Umsetzung von Impfempfehlungen.

Patients with inflammatory rheumatic diseases are known to have an increased risk of infections due to the rheumatic disease itself and due to therapy with immunosuppressive agents. The most important procedure to prevent infections is vaccinations, which are usually well-tolerated. The German National Commission for Immunization ( STIKO) has published recommendations for patients with an immunodeficiency. The German Society of Rheumatology (DGRh) has generally implemented these recommendations for patients with chronic inflammatory rheumatic diseases. The immunization status of patients with rheumatic diseases is of increasing importance in routine patient care because some of the recently approved drugs may influence the strength of the immune response to vaccination. However, there is almost no information about the current immunization status and the willingness of patients with rheumatic diseases to undergo vaccination procedures in Germany. There are also no epidemiologic data on the implementation of recommendations for immunization at the level of general practitioners. Here we present the results of a prospective study on the efficacy of standardized recommendations for immunization given to different patient groups with rheumatic diseases treated in a hospital specialized in rheumatology.

[Generalized osteoarthritis in a young adult with type II collagenopathy]. / Generalisierte Arthrose im jungen Erwachsenenalter bei Typ-II-Kollagenopathie.

HISTORY AND ADMISSION FINDINGS: In the 20-year-old patient increasing arthralgia since the age of seven years had caused functional impairment. As seronegative osteodestructive rheumatoid arthritis had been suspected, he was treated with methotrexate and steorid for ovemore than one year, without success.r INVESTIGATIONS: The clinical examination revealed a compromised function of the knee and hip joints. Conventional radiographs showed severe degenerative changes in these regions. The radiographs of the lumbar and the thoracic spine were suggestive of osteoporotic fractures. DIAGNOSIS, TREATMENT AND COURSE: The clinical and radiological findings led to the suspicion of a hereditary collagenous disease. Genetic analyses revealed a COL2A1 mutation. The treatment included analgesics, non-steroidal antirheumatic drugs, opiates and regular physiotherapy to build up and maintain muscle strength. CONCLUSION: In young adult patients with osteoarthritis mutations in the COL2A1 gene should considered. This mutation with the substitution pArg75Cys should be in focus because of the phenotypes in clinical manifestations.

[Perioperative use of biologicals in rheumatoid arthritis]. / Perioperativer Umgang mit Biologika bei rheumatoider Arthritis.

The high prevalence of patients with rheumatoid arthritis (RA) treated with biologics and the high proportion of RA patients requiring elective orthopaedic surgery indicates that the question of whether to continue or to interrupt biologic therapy in the context of a surgical procedure is a clinically relevant problem. Few data are available and the quality of the studies performed is somewhat limited. Thus, a straightforward recommendation on how this problem should be handled cannot be given on this basis. Therefore, individualized management based on risk stratification for postoperative complications seems currently to be the best solution.

[Arthritis of the atlanto-axial joint with inflammatory neck pain as a primary manifestation of seronegative rheumatoid arthritis]. / Atlantoaxiale Arthritis mit entzündlichem Nackenschmerz als Erstmanifestation einer seronegativen rheumatoiden Arthritis.

HISTORY AND ADMISSION FINDINGS: A 68-year-old woman with known degenerative joint disease suffered from increasing neck pain. Physical examination revealed painfully restricted movement of the cervical spine. INVESTIGATIONS: Erythrocyte sedimentation rate and C-reactive protein were increased. Tests for rheumatoid factors, antinuclear, anti-citrullinated protein and anti-neutrophil cytoplasmic antibody were negative. Cervical spine x-ray showed osteochondrosis with partially bridging spondylosis at C5/C6, but there was no atlanto-axial dislocation. Magnetic resonance imaging (MRI) revealed bone marrow edema and hyperintensity of the odontoid process, but there were no indications of fissures or fracture lines. TREATMENT AND COURSE: These findings indicated seronegative rheumatoid arthritis, with predominantly active atlanto-axial arthritis. After methotrexate and prednisolone had been administered the symptoms improved rapidly and inflammatory parameters returned to normal. Three months later no atlanto-axial arthritis was seen at MRI. CONCLUSION: Rheumatoid arthritis involving the atlanto-axial region should be considered in patients with persisting neck pain and signs of inflammation.

Successful use of anakinra to treat refractory Schnitzler's syndrome.

Schnitzler's syndrome is a rather rare disease which may appear in a rheumatologist's office because patients often report rheumatic symptoms with joint, bone and muscle pain. However, it is characterized by chronic urticaria, recurrent fever, liver and spleen enlargement, osteosclerosis, and lymphadenopathy, in conjunction with a serum IgM M component. A patient who had been treated with relatively high doses of corticosteroids for 10 years with insufficient response was treated with the IL-1 receptor antagonist anakinra, this led to a complete resolution of symptoms.

[Successful treatment of clinical manifestations of Muckle-Wells syndrome with anakinra]. / Erfolgreiche Behandlung der klinischen Manifestationen des Muckle-Wells-Syndroms mit Anakinra.

HISTORY: A 58-year-old woman and her 18-year-old daughter were first seen in our rheumatology centre after having experienced many years of periodic fever, arthralgia and urticaria. Some months ago a diagnosis of Muckle-Wells syndrome (MWS) had been made and confirmed by genetic testing. The mother had developed partial deafness and substantial loss of vision. Her daughter had been suffering mainly from urticaria and fever at least once daily. THERAPY: Because of the established role of interleukin (IL)-1 in this hereditary disease and some positive case reports, we decided to treat these patients with the IL-1 receptor antagonist anakinra. The patients reported a great success of this treatment with virtually complete absence of any acute MWS-associated symptoms. CONCLUSION: Therapy of MWS with anakinra seems to be highly efficacious for several clinical manifestations of this disease, including laboratory markers for inflammation. It is possible that organ destruction may be prevented by this medication.